13: Hematologic disorders Flashcards
(341 cards)
1
Q
hemophilia A factor
A
factor VIII deficiency
2
Q
factor VIII deficiency
A
hemophilia A
3
Q
hemophilia B factor and aka
A
factor IX deficiency
aka christmas dz
4
Q
factor IX deficiency
A
hemophilia B
5
Q
deep hemorrhage in muscles, soft tissues, joints
A
hemarthrosis
6
Q
hemorrhage in hemophilia causing a tumor like mass
A
pseudotumor of hemophilia
7
Q
plasminogen deficiency inheritance and mechanism
A
AR
fibrin build up in mucosa (plasminogen –> plasmin –> fibrin degradation)
8
Q
plaques in conjunctival mucosa in plasminogen deficiency
A
ligneous conjunctivitis
9
Q
ligneous conjunctivitis what and where
A
plaques in conjunctival mucosa in plasminogen deficiency
10
Q
confirmation test for plasminogen deficienct
A
Fraser-Lendrum stain of ligenous conjunctivities plaques in conjunctival mucosa
11
Q
Fraser-Lendrum
A
stain of ligenous conjunctivities plaques in conjunctival mucosa
confirmation test for plasminogen deficienct
12
Q
sickle cell anemia genetic underpinnings
and process generally
A
T–> A substitution
codes valine instead of glutamic acid
chronic hemolutic anemia
13
Q
sickle cell trait (vs dz)
A
trait – one gene affected
dz – both genes mutated
14
Q
sickle cell crisis presentation and precipitating factors
A
pain and fever
precipitated by hypoxia, infection, dehydration
15
Q
acute chest syndrome
A
lung involvement in sickle cell anemia
16
Q
lung involvement in sickle cell anemia
A
acute chest syndrome
17
Q
thalassemia what kind of process
A
hypochromic, microcytic anemia
18
Q
thalassemias differences and grades
A
beta:
minor - one gene
major – two genes, Coley’s anemia
chipmunk facies
alpha: one gene -- no dz two genes - trait three genes HbH disease four genes hydrops fetalis
19
Q
hydrops fetalis and thalassemia
A
alpha with 4 genes affected
20
Q
HbH disease
A
alpha thalassemia 3 genes
21
Q
coley’s anemia
A
beta thalassemia, major, two genes
22
Q
chipmunk face
A
beta thalassemia
23
Q
excess iron in tissues
A
hemochromatosis
24
Q
aplastic anemia
A
failure to produce all types of blood cells
25
failure to produce all types of blood cells
aplastic anemia
26
aplastic anemia associated with
Fanconi anemia and dyskeratosis congenita
27
dyskeratosis congenita heme implications
aplastic anemia, Fanconi anemia
28
people in certain regions are healthy but low on neutrophils
benign ethnic neutropenia
29
genetic neutropenia demographic and syndromes
```
infants
dyskeratosis congenita,
cartilage hair synrdomee
Schwachman-Diamond syndrome
severe congenital neutropenia
```
30
acquired neutropenia demographic and dzz
adults
| leukemia, Gaucher dz, osteopetrosis, drugs, vit B12 def, hepatitis
31
agranulocytosis most affected cells
neutrophils
32
kostmann sydndrome underlying mechanism
reduced granulocyte colony stimulating factor (G-CSF)
33
reduced granulocyte colony stimulating factor (G-CSF) sybdrome
kostmann syndrome
34
cyclic neutropenia genetics
neutrophil elastase (ELA2) gene mutation
35
neutrophil elastase (ELA2) gene mutation
cyclic neutropenia
36
thrombocytopenia 2 possible mechanisms
reduced platelets:
| reduced production or increased destruction
37
thrombotic thrombocytopenia purpura
increased use of plts in abnormal clot formation
38
increased use of plts in abnormal clot formation
thrombotic thrombocytopenia purpura
39
idiopathic thrombocytopenia purpura
childhood after viral infx, resolves spontaneously
40
thrombocytopenia in children after viral infx
idiopathic thrombocytopenia purpura
| resolves spontaneously
41
polycythemia vera what is and genetics
increase in mass of RBC
| JAK2 mutation
42
JAK2 mutation in heme
polycythemia vera
| increase in mass of RBS
43
painful burning with erythema of hand and feet in polycythemia vera
erythromelalgia
44
erythromelalgia
painful burning with erythema of hand and feet in polycythemia vera
45
syndromes with higher risk of leukemia
down, NF1, Klinefelter, Fanconi anemia
46
philadelphia chromosome which and where and gene product
chronic myeloid leukemia (22q) translocation
| bcr-abl gene
47
CML genetics
(22q) translocation -- Philadelphia chromosome
| bcr-abl gene
48
bcr-abl gene
CML
| (22q) translocation -- Philadelphia chromosome
49
22q translocation in hemepath
chronic myeloid leukemia (22q) translocation (philadelphia)
| bcr-abl gene
50
tumor like growth from leukemic infiltrate in soft tissues
granulocytic sarcoma or extramedullary myeloid tumor or chloroma
51
chloroma
tumor like growth from leukemic infiltrate in soft tissues
aka granulocytic sarcoma or extramedullary myeloid tumor
52
extramedullary myeloid tumor
tumor like growth from leukemic infiltrate in soft tissues
aka granulocytic sarcoma or chloroma
53
histiocytosis X what is and possible presentations
eosinophilic granuloma of bone
chronic disseminated histiocytosis: Hand-Schuler-Christian dz
acute disseminated histiocytosis: Leferer-Siwe disease
54
chronic disseminated histiocytosis
Hand-Schuler-Christian dz
eosinophilic granuloma of bone
histiocytosis X
55
Hand-Schuler-Christian dz
chronic disseminated histiocytosis
eosinophilic granuloma of bone
histiocytosis X
56
eosinophilic granuloma of bone
histiocytosis X
chronic disseminated histiocytosis: Hand-Schuler-Christian dz
acute disseminated histiocytosis: Leferer-Siwe disease
57
acute disseminated histiocytosis:
Leferer-Siwe disease
eosinophilic granuloma of bone
histiocytosis X
58
Leferer-Siwe disease
acute disseminated histiocytosis
eosinophilic granuloma of bone
histiocytosis X
59
histiocytosis X IHC
CD1a or CD207 (+++)
60
non hodgkin lymphoma higher prevalence in what conditions
immune problems:
| AIDS, Sjogrens, SLE, RA, organ transplant, Bloom syndrome
61
mycosis fungoides what is and what from
cutaneous T cell lymphoma
| from CD4+ T helper lymphocytes
62
cutaneous T cell lymphoma
| aka and what from
mycosis fungoides
| from CD4+ T helper lymphocytes
63
stages of mycosis fungoides
eczematous, plaque, tumor
64
sezary syndrome
aggressive mycosis fungoides that is really a T cell leukemia affecting multiple organs
65
aggressive mycosis fungoides that is really a T cell leukemia affecting multiple organs
sezary syndrome
66
sezary cells
atypical lymphocytes
67
lymphocytes in epithelium in hemepath
Pautrier's microabscesses
68
burkitt's lymphoma genetics
8:14 translocation
| c-myc overexpression
69
8:14 translocation
burkitt's lymphoma
| c-myc overexpression
70
c-myc overexpression
burkitt's lymphoma
| 8:14 translocation
71
bence jones proteins
light chain proteins produced in multiple myeloma --> renal falure
72
renal failure in multiple myeloma why
bence jones proteins (light chain)
73
most common site for plasmacytoma
spine
74
acute vs chronic LAD
acute: tender, soft, freely movable
chronic: non tender, rubbery, freely movable -- ddx from lymphoma which progresses
75
what tonsil look would be concerning
asymmetrical enlargement
76
phagocytized material in m/ph c/pl
tingible bodies
77
tingible bodies
phagocytized material in m/ph c/pl
78
most common hemophilia, what's wrong , labs
A -- f VIII defcy (85% cases)
| increased PTT
79
hemophilia B whats wrong, labs
aka christmas dz
f IX defcy
increased PTT
80
hemophilia vs vWF dz
hemophilia PTT increase
| vWF PTT and BT increase
81
most common inherited bleeding disorder
vWF disease
82
common initial sign of hemophilia
failure of normal hemostasis after circumcision
83
infants with hemophilia oral signs
oral laceration and ecchymoses on lips and tongue
84
how much factor VIII is enough for normal function
>25% normal
<5% mild symptoms
severe <1%
85
deep hemorrhage in hemophilia where, name, consequences
hemarthrosis
in mm, ST, joints (kness) --> deformity of joints
--> psedotumor of hemophilia
86
which coag pathway PTT shows
intrinsic
87
prothrombin function and pathwat
extrinsic
| measures presence/absence of clotting factors I, II, V, VII, X
88
plasminogen inheritance
AR but more common in F
89
aka for plasminogen deficiency
ligneous conjunctivitis
90
what does ligneous mean
wood-like
91
oral plasminogen deficiency
patchy ulcerated papules and nodules on gingiva; wax and wane
92
plasminogen deficiency histo
acellular eosinophilic material
congo red negative
fraser lendrum confirms it's fibrin
93
plasminogen deficiency mucosal surfaces involved
conjunctival mucosa (upper eyelid), oral, laryngeal (hoarseness), vaginal
94
anemia first physical sign
paleness of mucous membranes (esp palpebral conjunctiva)
95
sickle cell anemia mechanis,
T-A substitution
valine coded instead of glutamic acid in beta globin chain of hemoglobin
hemoglobin molecule is then prone to aggregation and become rigid and curved like a sickle
96
sickle cell trait presentation
only one gene affected, 40-50% of Hb abnormal, no manifestations
97
protective effects of sickle cell
against malaria
98
sickle cell dz vs trait
dz bpth genes mutated
99
consequences of sickle cell anemia
RBCs block capillaries --> chronic hemolytic anemia, ischemia, infarction
100
precipitating factors for sickle cell crisis
how presents, what's affected, how long
hypoxia, infection, hypothermia, dehydration
pain and fever; extreme pain from infarction of bones, lung, abdomen
lasts 3-10 days
101
acute chest syndrome
lung involvement in sickle cell crisis 2/2 fat embolism or pneumonia
102
susceptibility with sickle cell
S pneumoniae
| most common cause of death in children
103
sickle cell anemia on dental xrays
rarefaction of md and mx, enlarged marrow spaces
coarse stepladder bone trabeculae (bone marrow hyperplasia and increased hematopoiesis)
hair-on-end skull raadiograph
104
hair-on-end skull raadiograph in a kid
sickle cell and thalassemia
105
reduced trabecular pattern in a kid
sickle cell and thalassemia
106
thalassemia what kind of anemia
hypochrmoic, microcytic 2/2 hemolysis of abnormal RBCs
107
minor beta thalassemia manifest
one gene affected, no manifestations
108
major beta thalassemia aka, manifest
Cooley's or mediterranean anemia
two genes affected
growth of mx/md (chipmunk facies)
bone marrow hyperplasia (increased hematopoiesis with reduced trabecular pattern_, hepatosplenomegaly, hair on end
109
alpha thalassemia grades of intensity
one gene no disease
two genes trait (mild anemia)
three genes - HbH disease (hemolytic anemia and splenomegaly)
four genes - hydrops fetalis (generalized fetal edema)
110
hydrops fetalis what is and why
generalized fetal edema
| four gene alpha thalassemia
111
HbH dz what is and why
hemolytic anemia and splenomegaly
| three gene alpha thalassemia
112
two gene alpha thalassemia how look
mild anemia
113
thalassemia therapy complication
hemochromatosis -- buildup of iron after repeated transfusions
114
aplastic anemia what is and why
BM fails to produce all types of blood cells
| autoimmune pancytopenia
115
triggers of aplastic anemia
toxins (benzene), drugs (chloramphenicol), infection (hepatitis)
116
syndromes with increased frequency of aplastic anemia
Fanconi anemia and dyskeratosis congenita
117
aplastic anemia physical look
RBC def-cy --> pallor, tiredness
plt def --> bleeding
WBC def --> most significant; infections
118
oral look of aplastic anemia
petechiae, ecchymoses, pale mucosa, oral ulcerations, gingival hyperplasia
119
aplastic anemia histo
acellular marrow
120
accelular marrow on histo
aplastic anemia
121
neutropenia labs and consequences
n/ph below 1500/mm3
normal 2500-2600
increased susceptibility to infx
122
genetic neutropenia age and dzz
infants
| dyskeratosis congenita, cartilage-hair syndrome, schwachamn-diamond syndrome, severe congenital neutropenia
123
acquired neutropenia age and dzz
adults
leukemia, Gaucher, osteopetrosis, drugs (chemo, abx), vit B12 def, infections (He p a and b, HIV, measles, varicella, rubella, TB, typhoid)
124
neutropenia and infections (especially which); manifest as
bacterial infx (esp S aureus)' abscess formation reduced
middle ear, perirectal and oral infections (ulcerations on gingival mucosa)
125
cyclic neutropenia genetics, presentation, oral, diagnostic criteria
neutrophil elastase (ELA2) gene mutation
18-21 day cycle signs/symptoms
oral: mucosal ulcerations (most severe in gingiva); +/- bone less
dx: sequential CBC (2x/week for 8 weeks) -- must be <500 mm3 (3-5 days, 3 cycles)
126
agranulocytosis which ones are particularly absent
n/ph
127
kostmann syndrome
congenital agranulocytosis, low granulocyte colony stimulating factor
128
congenital agranulocytosis, low granulocyte colony stimulating factor
kostmann syndrome
129
kostmann syndrome presentation
ulcers of oral mucosa
gingivitis and gingival necrosis (NUG-like)
periodontitis
decreased hematocrit
130
oral ulcers, gingivitis/periodontitis, decreased hematocrit
kostmann syndrome
| congenital agranulocytosis, low granulocyte colony stimulating factor
131
normal plts and at which count signs of trombocytopenia show
normal 200k-400k
| signs at <100k/mm3
132
causes of thrombocytopenia
reduced production )BM cancer, CT)
increased destruction (drugs, most common heparin)
sequestration in spleen (gaucher dz, portal HTN)
133
increased destruction of plts which conditions
SLE and HIV
134
idiopathic (immune) thrombocytopenic purpura
| who, why, how long
childhood after viral infecion
| resolves spontaneously in 4-6 wks (max 6 mos)
135
tx for childhood purpura
idiopathic (immune) thrombocytopenic purpura
| resolves spontaneously in 4-6 wks (max 6 mos)
136
purpura after viral infection
idiopathic (immune) thrombocytopenic purpura
137
abnormal clot formation and increased consumpion of plts name and consequences
thrombotic thrombocytopenic purpura
| numerous thrombi in small vessels
138
thrombocytopenia histo
gingival biopsy
| 30-40% show fibrin deposits (PAS+) in small vessels --> occlusion
139
polycythemia vera aka and what is
primary acquired erythrocytosis
increase in mass of RBC
uncontrolled production of plts and granulocytes also
140
polycythemia vera genetics
JAK2 mutation
141
skin complaints polycythemia vera
generalized itching after bath without rash
| erythromelalgia: painful burning with erythema and warmth of hand and feet
142
oral and systemic complaints in polycythemia vera
gingival hemorrhage and myocardial infarction
143
chronic granulomatous disease of childhood aka and what i s
bridges-good syndrome, chronic gramulomatous disorder, Quie sundrpme
NADPH oxidase enzyme defect -- PMNs can't destroy bacteria
144
chronic granulomatous disease of childhood what happens and how diagnose
gramulomata form in many organs
chronic oral ulcerative lesions
dx: nitroblue-tetrazolium test
145
nitroblue-tetrazolium test for what
chronic granulomatous disease of childhood
| NADPH oxidase enzyme defect -- PMNs can't destroy bacteria
146
chronic granulomatous disease of childhood msot common infections
pneumonia, abscesses, suppurative arthritis, osteomyelitis, bacteremia/fungemia, superficial skin infections (cellulitis or impetigo)
147
child with chronic oral ulcers and multiple abscesses suspect what and how test
chronic gramulomatous disease
NADPH oxidase enzyme defect -- PMNs can't destroy bacteria
dx: nitroblue-tetrazolium test
148
leukemia associated with which conditions
Down, Bloom, NF1, Schwachman-Diamond, ataxia-telangietasia, klinefelter, Fanconi anemia, Wiskott-Aldrich
149
environmental risks for leukemia
chemicals, radiation, viruses (HTLV_1)
150
acute myeloid leukemia age range and survival
broad range
| 40% survival
151
acute lymphoblastic leukemia age range and survival
children, 80% survival
152
chronic myeloid leukemia age range and survival
adults 0 80% survival
153
chronic lymphocytic leukemia age range and survival
older adults; incurable but variable course
154
philadelphia chromosome what, which dz, prodycts
CML
22;9
bcr-abl gene and a chimeric protein with tyrosine kinase activity
155
which heme malignancy produces a chimeric tyrosine kinase
CML
22;9 -- philadelphia chromosome
bcr-abl gene and a chimeric protein with tyrosine kinase activity
156
22;9 chrom
CML
philadelphia
bcr-abl
tyrosine kinase
157
early stage AML name
myelodysplasia syndromes
158
leukemia oral look
ulcerations (esp gingival -- depp, punched out), candidiasis, herpes
159
chloroma what is and where most common (site and condition)
tumor like growth from leukemic infiltrate in soft tissues
gingival infiltration most seen in AML
160
blast transformation where and what happens
in CML
| cells become less differentiated, proliferate and --> death in 3-6 months
161
induction chemo vs maintenance chemo
induction: high doses to destroy cancer cells --> remission
| maintenance -- low --> maintain remission
162
richter syndrome
CLL becoming large cell lymphoma
163
CLL becoming large cell lymphoma
richter syndrome
164
tumor like growth from leukemic infiltrate in soft tissues
chloroma
gingival infiltration most seen in AML
165
leukemia with the most gingival infiltration
AML
166
forms of langerhans cell histiocytosis
eosinophilic granuloma of bone (mono or polyostotic)
chronic disseminated histiocytosis (Hand-Schuler-Chritian dz)
acute disseminated histiocytosis (Leferer-Siwe)
167
hand-schuler-christian dz other name and signs
chronic disseminated histiocytosus/LCH
| bone lesions, exophthalmos, diabetes insipidus
168
LCH xray
punched out RL, scooped out, teeth floating in air, most in post MD
169
punched out RL, scooped out, teeth floating in air, most in post MD
LCH
170
adult LCH ddx
can mimic severe localized periodontal disease in adult
171
birbeck granules
rod shaped structures in c/pl of langerhans cells on EM
172
rod shaped structures in c/pl of langerhans cells on EM
birbeck granules
173
langerhans cell histiocytosis IHC
```
CD1a or CD207 (+++)
peanut agglutinin (PNA)
```
174
microorganism with hodgkins lymphoma
EBV
175
age and location for hodgkin
75% in cervical and supraclavicular LNs
| 15-35 and 50+
176
typical presentation of hodgkin lymphoma
nontender LAD -- progresses from one LN chain to another
| weight loss, fever, night sweats, pruritus
177
histo types of hodgkin lymphoma
nodular lymphocyte predominant (popcorn cells, good prognosis)
classical (lymphocyte rich; nodular sclerosis - 80%, more in F, 2nd decade, Reed Sternberg, lacunar cells, good prognosis; mixed cellularity - 20%, eosinophils present; lymphocyte depletion - most aggressive; unclassifiable)
178
popcorn cells which malignancy
nodular lymphocyte predominant hodgkin lymphoma
| good prognosis
179
nodular sclerosis demographic and what is
classical subtype of hidgkin lymphome
80% (most common ig)
more in F 2nd decade
Reed Sternbers, lacunar cells, good prognosis
180
eosniphils present in which lymphoma
hodgkin classical mixed cellularity
181
most aggressive hidgkin lymphoma
classical lymphocyte depleted
182
reed sternberg which malignancy and IHC
classicla hodgkin lymphoma
owl eye or pennies on a plate
CD15+ and CD30++
183
treatment scheme for hodgkin
ABVD
| adriamycin, bleomycin, vinblastine, dacarbazine
184
owl eye cell
Reed Sternberg, classical Hodgkin
185
how does non-hodgkin lymphoma grow
solid mass
186
nonhodgkin lymphoma predisposing conditions
immune problems:
| AIDS, Sjohrens, SLE, RA, organ tranplant, Bloom syndrome
187
infectious agents and lymphoms
EBV -- Hodgkin and Burkitt
| H pylori - MALT lymphoma of stomach
188
most common lymphomas of oral
Non Hodgkin
usually extranodal
DLBCL (high grade) most common, then follicular
189
most common oral site for lymphoma
waldeyers ring, then hard palate
190
most common lymphoma of salivary glands
| and histo look
follicular
| dumbbell shaped, back to back follicles (little mantle)
191
prognostic histo factor in follocular lymphoma
rati of centroblasts to centrocytes
192
IHC DLBCL
| + genetics
CD5 +/-
CD10 +/-
CD19 +
CD20 +
translocation 3q27 and t(14:18)
193
t(14:18)
DLBCL and follicular lymphoma
194
CD5 +/-
CD10 +/-
CD19 +
CD20 +
DLBCL
195
IHC follicular lymphoma and genetics
```
CD5 -
CD10 +
CD19+
CD20+
t(14;18), bcl2 overexpression
```
196
bcl2 overexpression
follicular lymphoma
197
SLL/CLL IHC
CD5 +
CD19 +
CD20+
CD23 +
198
CD5 +
CD19 +
CD20+
CD23 +
SLL/CLL
199
MCL IHC and genetics
CD5+
CD19+
CD20+
CD23 -
t(11;14)
200
CD5+
CD19+
CD20+
CD23 -
MCL
201
t(11;14)
MCL
202
MALT IHC
CD10 -
CD20+
CD21+
CD22 +
203
CD10 -
CD20+
CD21+
CD22 +
MALT
204
monoclonality techniques
IHC light chains;
gene rearrangement studies
flow cytometry
205
most common peripheral T cell lymphoma
angioimmunoblastic T cell lymphoma
| aka angioimmunoblastic T cell lymphadenopathy with dysproteinemia
206
angioimmunoblastic T cell lymphoma histo and main ddx
main ddx from Hodgkin
polymorphous lymph node infiltrate
marked increase in follicular dendritic cells and high endothelial venules
207
polymorphous lymph node infiltrate
| marked increase in follicular dendritic cells and high endothelial venules
angioimmunoblastic T cell lymphoma
208
angioimmunoblastic T cell lymphoma signs
fever, malaise, joint pain, skin rash
209
mycosis fungoides aka and cells of origin
cutaneous t cell lymphoma
| from t helper CD4+ lymphocytes
210
stages of mycosis fungoides
eczematous (histo v subtle; similar to psoriasis)
plaque (sezary cells in epith)
tumor (sezry cells in dermis and epidermis)
211
oral look of mycosis fungoides
erythematous, indurate dplaques or nodules, typically ulcerated
212
sezary syndrome
aggressive form of mycosis fungoides
| dermatopathic t cell leukemia affecting multiple organs (mostly kidney and liver)
213
aggressive form of mycosis fungoides
sezary syndrome
dermatopathic t cell leukemia affecting multiple organs (mostly kidney and liver)
214
sezary cells look and IHC
mycosis cells - atypical lymphocytes
cerebriform nucleus -- marked infolding of nuclear membrane
IHC CD4+
215
pautrier's microabscesses
aggregates of sezary cells in epithelium
| plaque stage of mycosis fungoides
216
aggregates of sezary cells in epithelium
pautrier's microabscesses
plaque stage of mycosis fungoides
217
tx for mycosis fungoides
antibody against CD52
218
types of Burkitt lymphoma
endemic (African, 95% EBV)
sporadic (American, 25% EBV)
immunodef-cy associated
219
demographic for endemic burkitt
boys (peak 7yo)
post mx more
young pts have more jaw involvement
220
early xray sign for burkitts
patchy loss of lamina dura
221
burkitt IHC
CD10+
| Ki67 almost 100%
222
burkitt histo look
starry sky
| reactive lightly stained histiocytes (tingible m/ph) in a background of dark tumor cells
223
starry sky
burkitt
224
burkitt molecular
t(8:14)
| c-MYC overexpression
225
t(8:14)
burkitt
| c-myc
226
c-myc
burkitt
| t(8:14)
227
extranodal NK/T cell lymphoma aka
angiocentric t cell lmphoma
228
extranodal NK/T cell lymphoma presentation
aggressive destruction of midline structures of palate and nasal fossa
deep necrotic ulcer in midlate palate with oronasal fistula
229
aggressive destruction of midline structures of palate and nasal fossa
deep necrotic ulcer in midlate palate with oronasal fistula
hemepath
extranodal NK/T cell lymphoma
230
lymphomatoid granulomatosis associated with and what is
extranodal NK/T cell lymphoma
| B cell proliferation induced by EBV
231
B cell proliferation induced by EBV
lymphomatoid granulomatosis
| extranodal NK/T cell lymphoma
232
extranodal NK/T cell lymphoma histo and IHC
mixed inflammatory cells around blood vessels (angiocentric
necrosis
CD56 and granzyme B
233
granzyme B stains in what
extranodal NK/T cell lymphoma
234
plasmacytoma what is and where
unifocal proliferatino of plasma cells
| most common site spine
235
plasmacytoma IHC
cyclin D1 and CD56+
236
extramedullary plasmacytoma vs classic
in soft tissues
90% head and neck
cyclin D1 and CD56 negative
237
plasmacytoma vs multiple myeloma
plasmacytoma less protein M
no bone marrow infiltration
no signs of anemia, hypercalcemia, or renal failure
238
plasmacytoma prognosis
50% develop MM in 3 years
| extramedullary only 30% tho
239
multiple myeloma demographic
most common heme malignancy in black ppl
| 2x common in black
240
most characteristic symptom of multiple myeloma
lumbar spine pain
241
cause of metastatic calcification in MM
hypercalcemia 2/2 tymor osteolysis
242
bence jones proteins what is and where
excess light chain protein (k or l) produced by MM cells
urine of 30-50% pts
can cause renal failure
243
characteristic deposits in oral cavity in MM patients
amyloid -- oral cavity and periorbital
| 15% MM patients
244
MM IHC
CD138, k and l to show monoclonality
245
monoclonal gammopathy
excess immunoglobulin (M protein) in serum and urine
246
MM what marks worse prognosis
high β2 microglobulin, low albumin, older pts
247
castleman's disease aka
angiofollicular lymph node hyperplasia
248
castleman disease what is
solitary or multicentric benign growth of lymphoid tissue in young pts (<30yo)
249
castleman disease histo look
node architecture obliteration with scattered depleted small follicaulr centers surrounded by a cuff of small lymphocytes (onion skin) divided by concentric rings of reticulin fibers resembling Hassall's corpuscles and penetrated by hyalinized vessels
250
node architecture obliteration with scattered depleted small follicaulr centers surrounded by a cuff of small lymphocytes (onion skin)
castleman disease
251
castleman disease systemic form clinical and syndromic association
```
adenopathy, fever, anemia, elevated ESR and hypergammaglobulinemia
older patietns with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes)
poor prognosis (assoc w lymphoma)
```
252
cause of castleman dz
HHV8
253
Kikuchi Fujimoto dz aka and what is
histiocytic necrotizing lymphadenitis
self limiting benign inflamm condition affecting cervical lymp nodes
254
kikuchi fujimotot demographic
asians, F>M
255
Kikuchi Fujimoto presentation
fever and leukopenia
256
Kikuchi Fujimoto histo and patterns
three patterns: proliferative, necrotic, xanthomatous
necrosis surrounded by histiocytes, lymphocytes, plasma cells
no neutrophils
257
rosai dorfmann dz aka and where
sinus histiocytosis with massive lymphadenopathy
90% in cervical LN
resolves spontaneously
258
rosai dorfmann what predisposes
pts with autoimmune lympoproliferative syndrome with Fas mutation
259
emperipolesis what condition and what is
rosai dorfmann dz
lymphocytophagocytosis
lymphocytes inside histiocytes
often forming wreath-like rings within cytoplasm
plasma cells, n/ph, RBC may be found inside histiocytes
also seen in cutaneous t cell lymphoma
260
rosai dorfmann IHC
S100+, CD68+
| CD1a neg
261
kawasaki dz aka
mucocutaneous lymph node syndrome
acute febrile vasculitic syndrome of early childhood
262
kawasaki HLA
BW22
263
leading cause of acquired heart dz in children
kawasaki dz
also risk factor for adult ischemic heart dz
264
kawasaki presentation
high fever 1-2 weeks
bilateral congestion of ocular conjunctivae
dryness, redness, fissuring of lips
diffuse reddening of oral and pharyngeal mucosa
LAD (necrotic lymph node)
oral -- strawberry tongue
265
high fever 1-2 weeks
bilateral congestion of ocular conjunctivae
dryness, redness, fissuring of lips
diffuse reddening of oral and pharyngeal mucosa
LAD (necrotic lymph node)
oral -- strawberry tongue
kawasaki
266
angiolymphoid hyperplasia with eosiophilia aka
epithelioud hemangioma
267
angiolymphoid hyperplasia with eosiophilia demographic
frequent in H&N of young asian men
| can affect both sexes
268
angiolymphoid hyperplasia with eosiophilia presentation and mechanism
proliferation of small vessels lined by plump endothelial cells
report trauma, pulsatile sensation, pruritus
usually more superficial (dermis) --> multiple papules and nodules
other sites: upper lip, salivary glands, lungs, bone
269
kimura's dz demographic
h&n of young adult asian men (strong racial predilection)
270
kimura dz similar to what but how different
similar to angiolymphoid hyperplasia with eosiophilia
kimura deeper -- subqt tissue, salivary glands, LN
fever, malaise, LAD (ALHE has no symptoms)
endothelial cells not very large and flatter (ALHE is more prominent vascular condition)
can show germinal centers (rare in ALHE)
271
kimura dz labs
peripheral blood eosinophilia and elevated IgE
272
mutation in sickle cell
thymine (glutamic acid) for adenine (valine) in DNA in beta-globin chain
273
step ladder differential
sickle cell or myxoma; depends on extent
274
which dzz provide resistance to malaria
thalassemia, sickle cell
275
under how many neuts pulmonary infection
<500
276
hemophilia bleeding test
PTT increased
277
vWF dz bleeding test
PTT, PFA/bleeding time increased
278
warfarin bleeding test
PT increased
279
aspirin bleeding test
bleeding time/PFA increased
280
PT factors
1, 2, 5, 7, 10
281
PTT factors
2, 5, 8-12
282
diagnosis of cyclic neutropenia
<500 for at least 3 successive cycles
283
thrombocytopenia when first evident
<100,000
284
thrombocytopenia when severe
<10,000
285
ADAMTS3 function
cleaves vWF
| deficient in thrombotic thrombocytopenic purpura
286
immune thrombocytopenic purpura acute vs chronic
Acute : after a viral infection in kids, resolves within 4-6 wks
Chronic: woman 20-40 yrs
287
early stages of AML
myelodysplasia syndromes
288
myelodysplasia syndromes
early stages of AML
289
reduction in number of RBC and WBC due to crowding of BM by malignant cells
Myelophthisic anemia:
290
Myelophthisic anemia:
reduction in number of RBC and WBC due to crowding of BM by malignant cells
291
tumor like keukemia
chloroma/granulocytic sarcoma/myeloid sarcoma
292
imatinib
tyrosine kinase inhibitor for CML
293
2nd gen tyrosine kinase inhibitor
imatinim first
| nilotinib 2nd
294
monoclonal antobody to CD20
rituximab
295
rituximab
monoclonal antobody to CD20
296
chronic LCH triad
diabetes insipidus 2/2 kidney low ADH
exophthalmos
bone lesions
297
5 types of classic hodgkin
```
Lymphocytic rich (6%)
o Nodular sclerosis (60%)
o Mixed cellularity (15-30%)
o Lymphocyte depletion (1%)
o Non classifiable
```
298
hidgkin that's not classic
nodule lymphocyte predominant
299
lymphoma treatment regimens
ABVD: Adrimycin, Bleomycin, Vinblastine, Dacarbazine ( used mostly )
MOPP: Mechlorethamine, Oncovine, Procarbazine, Prednisone ( not used anymore due
complications )
300
Mycosis Fungoides what is actually
(cutaneous T-cell Lymphoma)
301
stages of mycosis fungoides
Eczematous, Plaque, Tumor stage:
302
special tropic feature of mycosis fungoides
Epidermotropism: propensity to invade the epidermis of the skin
303
aggressive form of Mycosis fungoides
Sezary syndrome:
304
atypical cells in mycosis fungoides
Sezary cells or mycosis cells
305
aggregation of lymphocytes in mycosis fungoides
Pautrier microabscesses:
306
nuclei of lymphocytes in mycosis fungoides
Cerebriform nucleus: Abnormal nucleus ( folding of nuclear membrane)
307
Pautrier microabscesses which stage
mycosis fungoides plaque stage
308
treatment for mycosis fungoides
Photochemotherapy PUVA
Monoclonal Antibody CD52
Denileukin diftitix : diphtheria toxin targets IL-2 receptors
Extracorpeal photopheresis ingestion of 8-methoxypsoralen, irradiate WBC outside body
309
EBV related processes (6)
```
Mono
Oral Hairy Leukoplakia
Burkitt Lymphoma
Nasopharyngeal Carcinoma
Lymphoepithelial carcinoma
Hepatocellular carcinoma
```
310
burkitt genetics
t(8:14) (q24;q32)
| c-myc
311
t(8:14)
burkitt , c myc
312
Ki67 in burkitt
almost 100%
313
classic histo look of burkitt
starry sky 2/2 histiocytes with abundant c/pl
| night sky is the dark staining lymphoma cells
314
types of burkitt
African-EBV related in jaws of children
2. Endemic
3. Sporadic-American and abdominal mass
4. HIV/Immunodeficiency
315
tx for burkitt
Treatment: High dose Cyclophosphamide chemotherapy an alkylating agent of the
nitrogen mustard type (specifically, the oxazaphosphorine group)
316
polycythemia genetics
JAK 2
317
JAK 2
polycythemia
318
CML genetics
22+9 /BCR-ABL
319
BCR-ABL
CML 22+9
320
22+9
CML BCR-ABL
321
cyclic neutropenia genetics
ELA-2 /ELANE
322
ELA-2
cyclic neutropenia
323
TTP genetics
ADAMTS13
324
ADAMTS13
TTP
325
most chatacteristic presenting signs (2) in multiple myeloma
lumbar pain and kidney 2/2 bence jones light chains
326
how many multiple myeloma pts get amyloid
15%
327
amyloid stains
congo red, crystal violet
328
primary amyloidosis amyloid
AL
329
multiple myeloma amyloid
AL
330
secondary amyloidosis amyloid
AA
331
hemodialysis amyloid
Aβ2 M
332
AL amyloid which conditions
primary amyloidosis and MM
333
AA amyloid which condition
secondary
334
Aβ2 M amyloid which condition
hemodialysis
335
IHC exytamedullary plasmacytoma vs MM
weak or negative for cyclin D1 and CD56
336
radiation dose for plasmacytoma
at least 40Gy
337
iron deficiency anemia sybdrome
Plummer Vinson
338
pernicious anemia aka and causes
```
aka megaloblastic
decreased B12 (cobalamin) and folic acid
```
339
microcytic anemia criteria and conditions
MCV ≤ 80fl
```
TAILS
Thalassemia
Anemia of chronic disease
Iron deficiency
Lead poisoning
Siderblastic anemia
```
340
normocytic anemia criteria and conditions
MCV 80-100 fl
```
TTP
Hemolysis
Osteopetrosis
Sickle cell
Pregnancy
Many other examples
```
341
macrocytic anemia criteria and conditions
MCV ≥100 fl
```
Megaloblastic anemia
(B12 or foliate deficiency)
Reticulocytosis
Hemolytic anemia
Chemo
```
Others : a few examples
Alcohol, Liver disease
Hypothyroidism, pregnancy
Aplastic anemia, MM,
